Azami Ayaka, Takano Yoshinao, Honda Michitaka, Todate Yukitoshi, Tada Takeshi, Waragai Mitsuru, Fukushima Daizo, Suzuki Nobuyasu, Sato Atai, Abe Tsuyoshi, Teranishi Yasushi, Sakuma Hideo
Dept. of Surgery, Southern Tohoku General Hospital.
Gan To Kagaku Ryoho. 2016 Nov;43(11):1409-1412.
A desmoplastic small round cell tumor(DSRCT)is a very rare malignant tumor that mainly occurs in the intra-abdominal cavity in young adults.This neoplasm has an extremely poor prognosis, with a clinical course characterized by rapid progression and metastasis.We present a 31-year-old man who presented with chief complaints of dysphagia, ataxic gait, and hoarseness.He first underwent surgical resection of a tumor in the medulla oblongata; however, the lesion was suspected to be a metastatic neoplasm.Following a thorough medical examination, the patient was diagnosed with retroperitoneal DSRCT with multiple metastatic lesions.He received multidisciplinary treatment including debulking surgery for the primary lesion; radiotherapy for metastatic lesions in the brain, abdomen, and cervical lymph nodes; hepatic artery embolization for liver metastasis; and systemic chemotherapy.The patient died of progressive disease 17 months after the initial diagnosis.
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种非常罕见的恶性肿瘤，主要发生于年轻成年人的腹腔内。这种肿瘤预后极差，临床病程以快速进展和转移为特征。我们报告一名31岁男性，其主要症状为吞咽困难、共济失调步态和声音嘶哑。他首先接受了延髓肿瘤的手术切除；然而，该病变被怀疑为转移性肿瘤。经过全面的医学检查，患者被诊断为伴有多处转移灶的腹膜后DSRCT。他接受了多学科治疗，包括对原发灶的减瘤手术；对脑、腹部和颈部淋巴结转移灶的放疗；对肝转移灶的肝动脉栓塞；以及全身化疗。患者在初始诊断后17个月死于疾病进展。
